TY - JOUR
T1 - Everolimus and intensive behavioral therapy in an adolescent with tuberous sclerosis complex and severe behavior
AU - Gipson, Tanjala T.
AU - Jennett, Heather
AU - Wachtel, Lee
AU - Gregory, Mary
AU - Poretti, Andrea
AU - Johnston, Michael V.
N1 - Funding Information:
Supported by Grant 2K12NS001696-11A1 (to TTG) from the National Institute of Neurological Disorders and Stroke (NINDS) .
PY - 2013
Y1 - 2013
N2 - Background: Self-injury and aggression have been reported in individuals with TSC (tuberous sclerosis complex), yet few data exist about treatment. Everolimus, an mTOR inhibitor, has been FDA-approved for subependymal giant cell astrocytomas (SEGAs) and renal angiomyolipomas in TSC. However, clinical use of everolimus with direct, real-time observations of self-injury and aggression in an individual with TSC has not been reported. Methods: During an inpatient admission to a neurobehavioral unit, real-time measurements of behaviors and seizures were recorded. An interdisciplinary team used these data to make treatment decisions and applied behavioral and pharmacological treatments, one at a time, in order to evaluate their effects. Results: Aggression and self-injury improved with applied behavioral analysis (ABA), lithium, and asenapine. Improvements in SEGA size, facial angiofibromas, seizures, and the most stable low rates of self-injury were observed during the interval of treatment with everolimus. Conclusion: Mechanism-based treatments in the setting of an evidence-based behavioral and psychopharmacological intervention program may be a model with utility for characterization and treatment of individuals with severe behavior and TSC.
AB - Background: Self-injury and aggression have been reported in individuals with TSC (tuberous sclerosis complex), yet few data exist about treatment. Everolimus, an mTOR inhibitor, has been FDA-approved for subependymal giant cell astrocytomas (SEGAs) and renal angiomyolipomas in TSC. However, clinical use of everolimus with direct, real-time observations of self-injury and aggression in an individual with TSC has not been reported. Methods: During an inpatient admission to a neurobehavioral unit, real-time measurements of behaviors and seizures were recorded. An interdisciplinary team used these data to make treatment decisions and applied behavioral and pharmacological treatments, one at a time, in order to evaluate their effects. Results: Aggression and self-injury improved with applied behavioral analysis (ABA), lithium, and asenapine. Improvements in SEGA size, facial angiofibromas, seizures, and the most stable low rates of self-injury were observed during the interval of treatment with everolimus. Conclusion: Mechanism-based treatments in the setting of an evidence-based behavioral and psychopharmacological intervention program may be a model with utility for characterization and treatment of individuals with severe behavior and TSC.
KW - Aggression
KW - Behavioral intervention and epilepsy
KW - Everolimus
KW - Self-injury
KW - Tuberous sclerosis complex
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U2 - 10.1016/j.ebcr.2013.06.004
DO - 10.1016/j.ebcr.2013.06.004
M3 - Article
C2 - 25667844
AN - SCOPUS:84882983531
SN - 2213-3232
VL - 1
SP - 122
EP - 125
JO - Epilepsy and Behavior Case Reports
JF - Epilepsy and Behavior Case Reports
IS - 1
ER -